Lai Steven Y, Lo Amanda E, Wu Phillis
Hematology and Oncology, Olive View University of California Los Angeles (UCLA) Medical Center, Sylmar, USA.
Pathology, Olive View University of California Los Angeles (UCLA) Medical Center, Sylmar, USA.
Cureus. 2024 Dec 5;16(12):e75155. doi: 10.7759/cureus.75155. eCollection 2024 Dec.
Primary myelofibrosis (PMF) is an uncommon chronic myeloproliferative disorder that is commonly associated with Janus kinase 2 (JAK-2), calreticulin (CALR), or thrombopoietin receptor (MPL) mutations. Pre-fibrotic PMF (also known as pre-PMF or early PMF) is a subtype of PMF that is defined by a lower grade of fibrosis. In this report, we present a rare case of warm autoimmune hemolytic anemia (wAIHA) associated with pre-PMF. The patient is a 64-year-old female with chronic thrombocytosis who presented with wAIHA. She underwent treatment with high-dose steroids and intravenous immunoglobulin which was unsuccessful. Subsequent treatment with rituximab successfully caused her wAIHA to go into complete remission, without the need for maintenance therapy. A bone marrow biopsy showed pre-PMF. Her warm autoimmune hemolytic anemia was thought to be related to cytokine dysregulation and autoantibody formation, which is commonly seen in pre-PMF. Underlying myelofibrosis should be considered in a patient who presents with wAIHA and other hematologic abnormalities. In such cases, a bone marrow biopsy should be performed as part of the workup. Further investigation is required to determine the mechanism behind this association.
原发性骨髓纤维化（PMF）是一种罕见的慢性骨髓增殖性疾病，通常与Janus激酶2（JAK - 2）、钙网蛋白（CALR）或血小板生成素受体（MPL）突变相关。纤维化前期PMF（也称为pre - PMF或早期PMF）是PMF的一种亚型，其定义为纤维化程度较低。在本报告中，我们呈现了一例与纤维化前期PMF相关的罕见温抗体型自身免疫性溶血性贫血（wAIHA）病例。该患者为一名64岁女性，患有慢性血小板增多症，表现为wAIHA。她接受了大剂量类固醇和静脉注射免疫球蛋白治疗，但未成功。随后使用利妥昔单抗治疗成功使她的wAIHA完全缓解，无需维持治疗。骨髓活检显示为纤维化前期PMF。她的温抗体型自身免疫性溶血性贫血被认为与细胞因子失调和自身抗体形成有关，这在纤维化前期PMF中很常见。对于出现wAIHA和其他血液学异常的患者，应考虑潜在的骨髓纤维化。在这种情况下，骨髓活检应作为检查的一部分进行。需要进一步研究以确定这种关联背后的机制。
